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PubMedDate not supplied

Magnetic resonance imaging of hepatocellular carcinoma mimicking focal nodular hyperplasia: a potential pitfall in patients with cirrhosis?

The magnetic resonance imaging findings in 2 cases of pathologically proven hepatocellular carcinoma that mimicked focal nodular hyperplasia are presented. Both cases were found in patients with cirrhosis, a condition in which focal nodular hyperplasia is unlikely to occur. Recognition that hepatocellular carcinoma may mimic focal nodular hyperplasia in patients with cirrhosis may prevent misdiagnosis and allow for earlier intervention.

Aged↗
PubMed2005

Poliosis circumscripta associated with neurofibroma.

A 32-year-old woman presented at our clinic with a moderately soft, nontender, subcutaneous mass in the left temporal region and white hairs overlying the swelling. The lesion was surgically excised, and there was no recurrence at one year postoperatively. On histological examination, the hair follicles overlying the subcutaneous mass were devoid of pigment and a poorly demarcated fibrocellular process was seen in the subcutaneous mass, which was typical of neurofibroma. To our knowledge, only one case of poliosis associated with neurofibroma has been previously reported.

Adult↗
PubMed1988

Neurofibroma of the gallbladder seen as a papillary polyp.

A case of neurofibroma of the gallbladder in a 44-year-old woman without von Recklinghausen neurofibromatosis but with right hypochondralgia is reported. The tumor was seen preoperatively as a polypoid lesion and in the resected gallbladder as a papillary polypoid growth over the main intramural tumor mass. Microscopically, the tumor was identified as a neurofibroma with many tactile corpuscle-like structures (Wagner-Meissner bodies), which were found by electron microscopic and immunohistochemical examination to consist of Schwann cells. Twelve cases of non-epithelial neoplasms arising in the gallbladder are also reviewed.

Adult↗
PubMed1992

A novel glial growth inhibitory factor, gliostatin, derived from neurofibroma.

Neurofibroma tissue was investigated for the presence of glial growth modulators that would suppress the proliferation of glial cells. A novel endogenous polypeptide inhibitor of proliferation and DNA synthesis in glial cells, gliostatin, was purified from the extracts of neurofibroma by a procedure comprising dye and anion-exchange column chromatography, and HPLC. A monoclonal antibody raised against partially purified gliostatin showed no cross-reactivity with known cytokines, but adsorbed the growth inhibitory activity of gliostatin and immunochemically visualized the putative gliostatin bands on western blot analyses. Although the product showed an apparent M(r) of 100,000 accompanied by an inhibitory activity on gel filtration column chromatography, it migrated at a lower apparent M(r) of 50,000 under the reducing conditions on western blotting, indicating that a homodimeric structure of native gliostatin consisted of 50-kDa subcomponents. Gliostatin was a potent growth inhibitor acting at nanomolar concentrations against all glial tumor cells and glia maturation factor-stimulated astroblasts, but not neuronal cells.

Animals↗
PubMed1993

Growth-promoting action of adenosine-containing dinucleotide on neuroblastoma cells: detection of adenosine-cytidine dinucleotide (ApCp) in neurofibroma (NF1) extracts.

Neurofibroma type 1 tissue was investigated for the presence of growth-promoting activity on human neuroblastoma cells. The activity was isolated by gel filtration and reversed-phase column chromatographs from neurofibroma type 1 extracts. An adenosine-containing dinucleotide (adenylyl(3'-5')cytidine-3'-phosphate) was identified as one of the major components of the activities by its enzymatic fragmentation and liquid chromatography/mass spectrometry. Synthetic adenosine-containing dinucleotide derivatives such as cytidyl(3'-5')adenosine, cytidyl(2'-5')adenosine, adenylyl(3'-5')cytidine, and adenylyl(2'-5')cytidine showed a similar action. Cytidyl(3'-5')adenosine, cytidyl(2'-5')adenosine, and adenylyl(2'-5')cytidine, which are able to release a free adenosine through enzymatic hydrolysis, in particular elicited a strong activity corresponding to that of adenosine with the highest action. These results suggest that neuroblastoma cells are able to use adenosine-containing dinucleotides as well as mononucleotides for their survival and proliferation.

Adenosine↗
PubMed2002

Neurofibromas in NF1: Schwann cell origin and role of tumor environment.

Neurofibromatosis type 1 (NF1) is one of the most prevalent dominantly inherited genetic diseases of the nervous system. NF1 encodes a tumor suppressor whose functional loss results in the development of benign neurofibromas that can progress to malignancy. Neurofibromas are complex tumors composed of axonal processes, Schwann cells, fibroblasts, perineurial cells, and mast cells. Through use of a conditional (cre/lox) allele, we show that loss of NF1 in the Schwann cell lineage is sufficient to generate tumors. In addition, complete NF1-mediated tumorigenicity requires both a loss of NF1 in cells destined to become neoplastic as well as heterozygosity in non-neoplastic cells. The requirement for a permissive haploinsufficient environment to allow tumorigenesis may have therapeutic implications for NF1 and other familial cancers.

Alleles↗
PubMed2000

Malignant neurofibroma of the urinary bladder.

Malignant neurofibroma of the urinary bladder is a very rare entity and usually associated with von Recklinghausen's disease. We present the first case of sporadic malignant neurofibroma of the urinary bladder and a review of the literature.

Aged↗
PubMed1989

Solitary plexiform neurofibroma.

A 35-year-old man with a solitary subcutaneous cord running on the abdomen wall is reported. Histologic examination of the lesion permitted a diagnosis of plexiform neurofibroma. In this patient and in his family no other signs of von Recklinghausen's disease were found. The plexiform neurofibroma considered as pathognomonic of neurofibromatosis may occur in a solitary form.

Abdominal Muscles↗
PubMed2003

It takes two to tango: mast cell and Schwann cell interactions in neurofibromas.

Neurofibromas are benign tumors comprised primarily of Schwann cells and fibroblasts. Mast cell infiltration is a well-known phenomenon; however, their role in tumor pathogenesis has been enigmatic. In an elegant set of experiments using cells derived from a murine model of neurofibromatosis 1 (NF1), Yang et al. dissect the molecular pathways involved in mast cell migration to neurofibromin-deficient Schwann cells. These results set the stage for rational development of therapeutics that could influence the multicellular microenvironment of neurofibromas to inhibit the development and/or progression of these tumors in human NF1.

Alleles↗
PubMedDate not supplied

Endolaryngeal neurofibromas.

A neurofibroma in the larynx is quite unusual and is more likely to appear as a solitary, isolated lesion rather than a concomitant finding in von Recklinghausen's disease. A case is presented of a patient with residual endolaryngeal neurofibroma despite eight attempts at removal. The importance of complete extirpation during the initial operative procedure using frozen sections to guarantee clear surgical margins is advocated.

Aged↗
PubMed1986

Spinal neurofibromas: a report of 66 cases and a comparison with meningiomas.

A series of 66 spinal cord neurofibromas was analyzed for history, signs, surgical approach, and outcome. The tumors presented primarily with sensory symptoms. Plain films were abnormal in 1/2 of cases and 1/2 had a complete block. They were primarily intradural, and primarily thoracic. A conservative exam system was used for follow-up and 85% with pain had complete relief; 50% with motor loss had normal motor function, and 88% had normal sensation who had prior sensory loss. In comparison to meningiomas, the principal differences were that neurofibromas had an even sex distribution, a lower incidence of cord signs and symptoms, more frequent findings on plain x-rays, and higher cerebrospinal fluid protein. Surgical outcome was similar. Sacrifice of the involved root during removal usually did not produce a deficit. The series is compared with a similar series of meningiomas from the same institution over the same time period.

Adolescent↗
PubMed1994

Severe cervical kyphotic deformities in patients with plexiform neurofibromas: case report.

Two patients with cervical plexiform neurofibromas are presented. Each of these patients had severe cervical kyphosis and has undergone anterior decompression, anterior reconstruction, and posterior stabilization. We discuss the surgical management of cervical kyphotic deformities associated with plexiform neurofibromas and review the factors associated with bony changes in neurofibromatosis.

Adolescent↗
PubMed2000

Plexiform neurofibroma of the submandibular gland.

A rare plexiform neurofibroma of the submandibular gland in a patient with neurofibromatosis is presented. The clinical manifestations of the disease are reviewed. The need for early diagnosis of neurofibroma is emphasized because of malignant transformation.

Adult↗
PubMed2002

A solitary neurofibroma of the palatine tonsil.

A rare case of a tonsillar neurofibroma in a 28-year-old Caucasian male is presented. Benign tumours of the tonsils are rare and of those reported, only a few are benign peripheral nerve sheath tumours (BPNSTs). This is the first report of a solitary neurofibroma of the palatine tonsil in the English literature.

Adult↗
PubMed1997

Magnetic resonance imaging of subcutaneous diffuse neurofibroma.

A 31-year-old woman presented with increasing pain and tenderness of a long-standing soft tissue mass on her back. MRI showed a network of interconnecting tubular areas, which were T1 isointense and T2 hyperintense relative to skeletal muscle, and displayed marked Gd-DTPA enhancement. The lesion was situated within the subcutaneous fat. Clinically and radiologically, this mass was considered to be a subcutaneous venous haemangioma. Histological examination of the excised mass showed a diffuse neurofibroma with ectatic vessels and entrapped adipose tissue. Similar MRI appearances of subcutaneous haemangioma and diffuse neurofibroma may result in failure to make the correct diagnosis and in inappropriate management.

Adult↗
PubMed1987

Intraparotid facial nerve neurofibroma.

Neurogenic neoplasms of the intraparotid facial nerve are uncommon and are usually diagnosed intraoperatively by tissue biopsy. Fifty-six cases of primary neurogenic neoplasms involving the facial nerve have been reported. The majority of these have been schwannomas. A case of a solitary neurofibroma involving the main trunk of the facial nerve is presented. Schwannomas and neurofibromas have distinct histological features which must be considered prior to the management of these tumors. The management of neurogenic tumors associated with normal facial function is a particularly difficult problem. A new approach for the diagnosis and management of neurogenic neoplasms is described utilizing electroneurography.

Adult↗
PubMed1993

Clinical and pathological aspects of solitary spinal neurofibroma.

Four cases are described of solitary spinal neurofibroma, a rare tumour of the spinal cord or nerve roots. Computerised tomography provided an accurate topographical definition of the tumour. Magnetic resonance imaging showed an increased T2-weighted signal and multiple areas of decreased T1- and T2-weighted signals centrally. The MR signals matched the histological examination which showed hyperplastic interfascicular connective tissue, pleomorphic cells, and tightly packed nerve fibres compressed by the surrounding loose connective tissue. Electron microscopy showed three types of cell: Schwann cells, fibroblast-like cells, and mast cells. The histological findings suggests that solitary spinal neurofibroma is a distinct pathological entity which could be diagnosed preoperatively from the MR images.

Adult↗